Stephen J. Wort
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View article: Significance of Pulmonary Vascular Dysfunction in Chronic Obstructive Pulmonary Disease
Significance of Pulmonary Vascular Dysfunction in Chronic Obstructive Pulmonary Disease Open
Chronic obstructive pulmonary disease (COPD) is frequently accompanied by abnormalities of the pulmonary vasculature. This vasculopathy spans the spectrum from mild vascular dysfunction to pulmonary hypertension, which on rare occasions ca…
View article: Positioning Imatinib for Pulmonary Arterial Hypertension: A Dose-Finding Phase 2 Study
Positioning Imatinib for Pulmonary Arterial Hypertension: A Dose-Finding Phase 2 Study Open
Rationale: Imatinib, 400 mg daily, reduces pulmonary vascular resistance and improves exercise capacity in patients with pulmonary arterial hypertension. Concerns about safety and tolerability limit its use. Objectives: We sought to identi…
View article: Survival outcomes in EIF2AK4 mutation-associated pulmonary arterial hypertension: seeking clarity in contrast
Survival outcomes in EIF2AK4 mutation-associated pulmonary arterial hypertension: seeking clarity in contrast Open
Background Pulmonary veno-occlusive disease (PVOD) is a rare cause of pulmonary arterial hypertension (PAH) characterized by widespread fibrous intimal proliferation of pre-septal pulmonary venules and a lower lung diffusion capacity for c…
View article: Screening and diagnosis of pulmonary hypertension associated with chronic lung disease (PH‐CLD): A consensus statement from the pulmonary vascular research institute's innovative drug development initiative—group 3 pulmonary hypertension
Screening and diagnosis of pulmonary hypertension associated with chronic lung disease (PH‐CLD): A consensus statement from the pulmonary vascular research institute's innovative drug development initiative—group 3 pulmonary hypertension Open
Pulmonary hypertension (PH) is a frequent complication of chronic lung disease (CLD). However, PH is difficult to diagnose early since accompanying symptoms overlap and are similar to those of the underlying CLD. In most cases the PH is mi…
View article: Noninvasive diagnostic modalities and prediction models for detecting pulmonary hypertension associated with interstitial lung disease: a narrative review
Noninvasive diagnostic modalities and prediction models for detecting pulmonary hypertension associated with interstitial lung disease: a narrative review Open
Pulmonary hypertension (PH) is highly prevalent in patients with interstitial lung disease (ILD) and is associated with increased morbidity and mortality. Widely available noninvasive screening tools are warranted to identify patients at r…
View article: Sendaway capillary NT-proBNP in pulmonary hypertension
Sendaway capillary NT-proBNP in pulmonary hypertension Open
Background N-terminal pro-B-type natriuretic peptide (NT-proBNP) is a biomarker of cardiac ventricular wall stress that is incorporated into pulmonary hypertension (PH) risk stratification models. Sendaway sampling may enable patients to p…
View article: Blood DNA methylation profiling identifies cathepsin Z dysregulation in pulmonary arterial hypertension
Blood DNA methylation profiling identifies cathepsin Z dysregulation in pulmonary arterial hypertension Open
Pulmonary arterial hypertension (PAH) is characterised by pulmonary vascular remodelling causing premature death from right heart failure. Established DNA variants influence PAH risk, but susceptibility from epigenetic changes is unknown. …
View article: Pulmonary hypertension in the setting of interstitial lung disease: Approach to management and treatment. A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative—Group 3 Pulmonary Hypertension
Pulmonary hypertension in the setting of interstitial lung disease: Approach to management and treatment. A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative—Group 3 Pulmonary Hypertension Open
Pulmonary hypertension (PH) due to interstitial lung disease (ILD), a commonly encountered complication of fibrotic ILDs, is associated with significant morbidity and mortality. Until recently, the studies of pulmonary vasodilator therapy …
View article: The hepcidin-ferroportin axis influences mitochondrial function, proliferation, and migration in pulmonary artery endothelial and smooth muscle cells
The hepcidin-ferroportin axis influences mitochondrial function, proliferation, and migration in pulmonary artery endothelial and smooth muscle cells Open
Rationale Elevated circulating hepcidin levels have been reported in patients with pulmonary artery hypertension (PAH). Hepcidin has been shown to promote proliferation of human pulmonary artery smooth muscle cells (PASMCs) in vitro, sugge…
View article: Pulmonary hemodynamics and transplant‐free survival in sarcoidosis‐associated pulmonary hypertension: Results from an international registry
Pulmonary hemodynamics and transplant‐free survival in sarcoidosis‐associated pulmonary hypertension: Results from an international registry Open
Pulmonary hypertension (PH) is a risk factor for mortality in patients with sarcoidosis. Severe PH in chronic lung disease has previously been defined as mean pulmonary arterial pressure (mPAP) ≥ 35 mmHg or mPAP 25 ≥ mmHg with cardiac inde…
View article: Right ventricular functional recovery assessment with stress echocardiography and cardiopulmonary exercise testing after pulmonary embolism: a pilot prospective multicentre study
Right ventricular functional recovery assessment with stress echocardiography and cardiopulmonary exercise testing after pulmonary embolism: a pilot prospective multicentre study Open
Background Data on right ventricular (RV) exercise adaptation following acute intermediate and high-risk pulmonary embolism (PE) remain limited. This study aimed to evaluate the symptom burden, RV functional recovery during exercise and ca…
View article: Chaotic breathing in post-COVID-19 breathlessness: a key feature of dysfunctional breathing can be characterised objectively by approximate entropy
Chaotic breathing in post-COVID-19 breathlessness: a key feature of dysfunctional breathing can be characterised objectively by approximate entropy Open
Post-COVID-19 breathing pattern disorder can be characterised by application of nonlinear statistical modelling of exercise ventilatory data https://bit.ly/3WlBc7e.
View article: Beta-blockade improves right ventricular diastolic function in exercising pulmonary arterial hypertension
Beta-blockade improves right ventricular diastolic function in exercising pulmonary arterial hypertension Open
SCOPUS: le.j
View article: Pathogenesis, clinical features, and phenotypes of pulmonary hypertension associated with interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative ‐ Group 3 Pulmonary Hypertension
Pathogenesis, clinical features, and phenotypes of pulmonary hypertension associated with interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's Innovative Drug Development Initiative ‐ Group 3 Pulmonary Hypertension Open
Pulmonary hypertension (PH) is a frequent complication of interstitial lung disease (ILD). Although PH has mostly been described in idiopathic pulmonary fibrosis, it can manifest in association with many other forms of ILD. Associated path…
View article: Phosphodiesterase 5 inhibitor treatment and survival in interstitial lung disease pulmonary hypertension: A Bayesian retrospective observational cohort study
Phosphodiesterase 5 inhibitor treatment and survival in interstitial lung disease pulmonary hypertension: A Bayesian retrospective observational cohort study Open
Background and Objective Pulmonary hypertension is a life‐limiting complication of interstitial lung disease (ILD‐PH). We investigated whether treatment with phosphodiesterase 5 inhibitors (PDE5i) in patients with ILD‐PH was associated wit…
View article: First Genotype–Phenotype Study in TBX4 Syndrome: Gain-of-Function Mutations Causative for Lung Disease
First Genotype–Phenotype Study in TBX4 Syndrome: Gain-of-Function Mutations Causative for Lung Disease Open
Rationale: Despite the increased recognition of TBX4 (T-BOX transcription factor 4)-associated pulmonary arterial hypertension (PAH), genotype-phenotype associations are lacking and may provide important insights. Objectives: To compile an…
View article: Clinical significance of pulmonary hypertension in interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's innovative drug development initiative—Group 3 pulmonary hypertension
Clinical significance of pulmonary hypertension in interstitial lung disease: A consensus statement from the Pulmonary Vascular Research Institute's innovative drug development initiative—Group 3 pulmonary hypertension Open
Pulmonary hypertension (PH) has been linked to worse outcomes in chronic lung diseases. The presence of PH in the setting of underlying Interstitial Lung Disease (ILD) is strongly associated with decreased exercise and functional capacity,…
View article: First genotype-phenotype study in TBX4 syndrome: gain-of-function mutations causative for lung disease
First genotype-phenotype study in TBX4 syndrome: gain-of-function mutations causative for lung disease Open
Rationale Despite the increasing frequency of TBX4 -associated pulmonary arterial hypertension (PAH), genotype-phenotype associations are lacking and may provide important insights. Methods We assembled a multi-center cohort of 137 patient…
View article: The Effect of Borderline Pulmonary Hypertension on Survival in Chronic Lung Disease
The Effect of Borderline Pulmonary Hypertension on Survival in Chronic Lung Disease Open
Background: The impact of the new “borderline” hemodynamic class for pulmonary hypertension (PH) (mean pulmonary artery pressure [mPAP], 21–24 mm Hg and pulmonary vascular resistance, [PVR], ≥3 wood units, [WU]) in chronic obstructive pulm…
View article: Use of Pulmonary Arterial Hypertension Therapies in Patients with a Fontan Circulation: Current Practice Across the United Kingdom
Use of Pulmonary Arterial Hypertension Therapies in Patients with a Fontan Circulation: Current Practice Across the United Kingdom Open
Background The Fontan circulation is a successful operative strategy for abolishing cyanosis and chronic volume overload in patients with congenital heart disease with single ventricle physiology. “Fontan failure” is a major cause of poor …